• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.370-375
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• 2002

Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods : Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA-matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. Results : Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. Conclusion : HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.302-310
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1119-1126
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• 2009

Purpose:This study compares the maternal characteristics and birth outcomes of infants of Asian immigrant mothers from developing countries with those of the infants of Korean mothers. Methods:In this multicenter and retrospective study, Asian immigrant women who had delivered between January 2005 and June 2008 were enrolled from9 Medical Centers. In all, 333 births to Asian immigrant women from developing countries (Asian-Korean infants) were included in this study. In addition, sex-, birth year-, and gestational age-matched 333 neonates born to Korean mothers were selected as the control group (Korean infants). On the basis of the hospital data, we investigated the nationality, age, and medical history of the mothers and compared the incidence of congenital infection, Apgar score, weight, height, and head circumference of Asian-Korean infants with those of the Korean infants. Results:The average maternal age of Asian women from developing countries at birth term was 26.7 years, which was significantly lower than that of Korean women (30.8 years, P<0.05). The birth weight of Asian-Korean infants (2,869 g) was significantly smaller than that of Korean infants (2,995 g, P<0.05). There was a significant difference in the incidence of congenital syphilis infection between the Asian-Korean infants and Korean infants (5 cases vs. 0 case, P<0.05). Conclusion:There were significant differences in the perinatal outcomes between the Korean and Asian-Korean infants. A multicenter large-scaled study should be performed to analyze the perinatal outcomes of Asian-Korean infants.

• Journal of Chest Surgery
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• v.43 no.4
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• pp.375-380
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• 2010

Background: Primary cardiac tumors are extremely rare. The most common type are benign myxomas, and these are almost completely curable with early surgery. Malignant tumors, however, such as sarcomas, are difficult to remove surgically, and their prognosis is known to be poor. In this study, data on patients who had undergone surgical treatment of cardiac tumor in the authors' hospital were collected and analyzed. Material and Method: The subjects included 28 patients who had undergone surgical treatment of cardiac tumor from August 1993 to December 2008. Their medical records were reviewed and retrospectively analyzed. Result: The patients were aged from 20 to 76 years (mean age: $54.2{\pm}15.6$), and 11 were male (39%) and 17 female (61%). Fifteen of them (54%) underwent emergency surgery to improve heart failure symptoms. The most common preoperative symptom was dyspnea (15 cases, 54%). Preoperative echocardiography was performed on all the patients. The average size of the tumor as measured during the operation was $7.0{\pm}6.9cm$ (the average length of the long axis was 2∼40 cm), and the sites of tumor attachment were the interatrial septum (18 cases, 64%), the left atrium (9 cases, 32%), the mitral valve annulus (2 cases, 7%), and the left ventricle (2 cases, 7%). The operation was performed with an incision through both atria in all the patients, and a complete excision was made in 25 cases (89%). According to the biopsy results, there were 4 cases of sarcoma (14%), 1 case of lipoma (4%), and 23 cases of myxoma (82%). The three cases in which the tumors were not completely excised were sarcomas. No operative deaths occurred after the operations. Outpatient follow-up was possible for 24 cases (86%), with a mean follow-up period of $46.8{\pm}42.7$ months. Late death occurred in 3 of the 24 patients; each of these patients had sarcomas. Of these patients, the first had undergone two repeat surgeries, the second had metastatic sites removed, and the last had only chemotherapy. The average recurrence time was $12.7{\pm}10.8$ months, and the average metastasis time was $20.5{\pm}16.8$ months. Conclusion: Most cardiac tumors are benign myxomas. In principle, they should be surgically treated because they can create risks such as embolism, and can be radically treated when surgically removed. In most cases, however, malignant sarcomas are already considerably advanced with severe infiltration into the neighboring tissues at the time of diagnosis. The surgical removal of malignant sarcomas is known to be difficult because of the advanced stage and degree of infiltration. We suggest that excision of the removable portion of the tumor sites to alleviate symptoms such as heart failure can improve quality of life.

• Radiation Oncology Journal
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• v.17 no.1
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• pp.30-35
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• 1999

Purpose : To evaluate the significance of squamous cell carcinoma antigen (SCC) and carcinoembryonic antigen (CEA) as tumor markers in uterine cervix carcinoma. Materials and Methods : In 22 patients with histologically proven primary squamous cell carcinoma of uterine cervix, tumor volume was checked either by using MRI (in 20 patients) or ultrasound (in 2 patients). Pre-treatment serum SCC levels were checked in 22 patients and CEA levels in 21 patients. After curative radiotherapy, post-treatment SCC and CEA were checked regularly. Results : SCC was raised In 68.2$\%$ and CEA was raised in 19.0$\%$ before treatment. The coefficient of correlation between tumor volume and pre-reatment SCC was 0.59382 when one extremely deviated case was excluded. And there was no correlation between tumor volume and CEA. After the treatment, SCC was raised En 9.1$\%$ and CEA was raised in 4.8$\%$. In further follow up measurement, raise of SCC was associated with clinical relapse or persistence of disease. The specificity of raised SCC level in association with recurrent or persistent disease was 93.8$\%$ . The sensitivity in association with recurrent or persistent disease was 100$\%$. The positive predictive values was 85.7$\%$. The median lead time for recurrence was 1.2 months. Conclusions: Both SCC and CEA were good tumor markers for monitoring treatment effect in patients with raised pre-treatment levels. But the sensitivity of pretreatment CEA was low, while that of pretreatment SCC was high. And there was no additional gain by adding CEA measurements to SCC measurements.

• Radiation Oncology Journal
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• v.17 no.1
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• pp.78-83
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• 1999

Purpose : We have compared the characteristics of Siemens virtual wedge device with physical wedges for clinical application. Materials and Methods : We investigated the characteristics of virtual and physical wedges for various wedge angles (15, 30, 45, and 60$^{\circ}$) using 6- and 15MV photon beams. Wedge factors were measured in water using an ion chamber for various field sizes and depths. In case of virtual wedge device, as upper jaw moves during irradiation, wedge angles were estimated by accumulated doses. These measurements were performed at off-axis points perpendicular to the beam central axis in water for a 15cm${\times}$20cm radiation field size at the depth of loom. Surface doses without and with virtual or physical wedges were measured using a parallel plate ion chamber at surface. Field size was 15cm H20cm and a polystyrene phantom was used. Results : For various field sizes, virtual and physical wedge factors were changed by maximum 2.1% and 3.9%) , respectively. For various depths, virtual and physical wedge factors were changed by maximum 1.9% and 2.9%, respectively. No major difference was found between the virtual and physical wedge angles and the difference was within 0.5$^{\circ}$ . Suface dose with physical wedge was reduced by maximum 20% (x-ray beam :6 MV, wedge angle:45$^{\circ}$, 550: 80 cm) relative to one with virtual wedge or without wedge. Conclusions : Comparison of the characteristics of Siemens virtual wedge device with physical wedges was performed. Depth dependence of virtual wedge factor was smaller than that of physical wedge factor. Virtual and physical wedge factors were nearly independent of field sizes. The accuracy of virtual and physical wedge angles was excellent. Surface dose was found to be reduced using physical wedge.

• Radiation Oncology Journal
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• v.21 no.1
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• pp.100-106
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• 2003

Purpose : To develop a patients' setup verification tool (PSVT) to verify the alignment of the machine and the target isocenters, and the reproduclbility of patients' setup for three dimensional conformal radiotherapy (3DCRT) and intensity modulated radiotherapy (IMRT). The utilization of this system is evaluated through phantom and patient case studies. Materials and methods : We developed and clinically tested a new method for patients' setup verification, using digitally reconstructed radiography (DRR), simulation, porial and digital images. The PSVT system was networked to a Pentium PC for the transmission of the acquired images to the PC for analysis. To verify the alignment of the machine and target isocenters, orthogonal pairs of simulation images were used as verification images. Errors in the isocenter alignment were measured by comparing the verification images with DRR of CT Images. Orthogonal films were taken of all the patients once a week. These verification films were compared with the DRR were used for the treatment setup. By performing this procedure every treatment, using humanoid phantom and patient cases, the errors of localization can be analyzed, with adjustments made from the translation. The reproducibility of the patients' setup was verified using portal and digital images. Results : The PSVT system was developed to verify the alignment of the machine and the target isocenters, and the reproducibility of the patients' setup for 3DCRT and IMRT. The results show that the localization errors are 0.8$\pm$0.2 mm (AP) and 1.0$\pm$0.3 mm (Lateral) in the cases relating to the brain and 1.1$\pm$0.5 mm (AP) and 1.0$\pm$0.6 mm (Lateral) in the cases relating to the pelvis. The reproducibility of the patients' setup was verified by visualization, using real-time image acquisition, leading to the practical utilization of our software Conclusions : A PSVT system was developed for the verification of the alignment between machine and the target isocenters, and the reproduclbility of the patients' setup in 3DCRT and IMRT. With adjustment of the completed GUI-based algorithm, and a good quality DRR image, our software may be used for clinical applications.

• Radiation Oncology Journal
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• v.26 no.3
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• pp.135-141
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• 2008

Purpose: To evaluate the clinical outcomes and prognostic factors in retroperitoneal soft tissue sarcomas treated by postoperative radiotherapy. Materials and Methods: The records of 23 patients with retroperitoneal soft tissue sarcomas, who underwent postoperative radiotherapy between 1985 and 2003, were analyzed. The median follow-up period was 77 months (range, $8{\sim}240$ months). A total of 21 patients presented with primary disease, and two patients presented with recurrent disease. Liposarcomas and leiomyosarcomas represented 78% of the diagnosed tumor cases. Moreover, 17 cases were of high grade (grade 2 or 3). The median tumor size was 13 cm (range, $3{\sim}50\;cm$). Complete excision was achieved in 65% of patients. The median radiation dose was 50.4 Gy (range, 45.0 to 59.4 Gy), with conventional fractionation. Results: The 5-year overall, local recurrence-free, and distant metastasis-free survival rates were 68%, 58%, and 71%, respectively. Eleven patients experienced local recurrence, while 9 patients experienced distant metastasis. The most common site for distant metastasis was the liver. A univariate analysis revealed that adjacent organ invasion and age (>60 years) as the significant risk factors contributing to the prediction of poor overall survival. Moreover, multivariate analyses indicated that adjacent organ invasion remained significantly associated with a higher risk of death. In addition, patient age (>60 years) was the other identified risk factor for local recurrence by univariate and multivariate analyses. Except for one case of grade 3 diarrhea, no patient suffered grade 3 or higher complications. Conclusion: Our results were comparable to previous reports in that adjacent organ invasion and patient age (>60 years) were significant predictors of poor survival and tumor recurrence, respectively.

• Journal of Korean Academy of Nursing Administration
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• v.3 no.1
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• pp.119-133
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• 1997

Continuing shift service of clinical nurses can be not only the cause of occupational dissatisfaction by being connected with the change of circadian rhythm and the burden of duties to be applicable to such changes. But also of inviting the lowering of nursing quality by being affected to the resignation of the nurses as the threat to the health of the nurses. This study has selected 500 nurses at random under non-probability sampling who have been serving by shift in 7 general hospitals which have over 400 sickbeds for the purpose of cross-sectional survey design from Sep. 7 through 20, 1996. Standardized modification of the CMI has been used which was designed for Koreans with Cornell Medical Index developed by Broadman and his fellow workers as the study device. The structure of the device was composed of 35 questions on physical appeal(Chronbach's ${\alpha}=8507$) and 22 questions of mental appeal(Cronbach's ${\alpha}=.8166$ totalling 57 questions. The collected data has been computerrized by using SPSS. General character, present symptom, perceived symptom and others are sought by practical number and percentage, and the health condition comparison followed by general characters was conducted by t-test and ANOVA. The post test was by Duncan's test by the level of p<.05. 1) The items of the answer that they have the physical symptom presently by 50% or over of the nurses were as "Do you often have spells of severe dizziness", "Are your eyes often red or inflamed", "Does press or pain in the head often make like miserable", "Are your ankles often badly swollen", "Do pains in the back make it hard for you to keep up with your work". 2) The items of the answer by over 50% of the nurses as the mental symptom at present were "do you fell bad when criticized?", "Do you get angry when everything is against your will?", "do you get angry when ordered to do this and that?", "do you feel uneasy by such a trifle thing?", "do you tremble or are you freightened by sudden sound?". The mental and physical symptoms which have appeared presently in connection with the shift service have been agreed with each other. But the physical condition has been worse than the mental one. 3) In the physical health conditions followed by demosociological character, there were the significant differences by sex, religion and place of residence(p<.05), and in the mental health conditions, there were the significant differences by age, marital status, residence place and the required time for attending hospital(p<.05). 4) There was significant difference by the degree of satisfaction about the duty in both the physical and mental health conditions. In short, the higher the degree of duty satisfaction, the better the health conditions. 5) There were the significant difference according to the times of night duty and whether they take the drug or not or the kinds of the drugs in the physical health conditions related with the characters of night shift. Mental health conditions in the night shift case showed significant differences according to their taking drug or not or the kinds of the drugs(p<.05). I can confirm that the nurses have been affected continuously by the shift service mentally and physically. The maintenance of the physical and mental health of the nurses and its promotion are very important problem to guarantee the quality nursing in the performance of the nursing service continuously and effectively, so the hospital should make every effort to improve the duty conditions by finding out the causes affecting to their health. In the nursing management viewpoint, I think that elevating the satisfaction degree about the duty would be a great help to the promotion of physical and mental health conditions. But what is most important is that the nurses themselves should take care of themselves in maintaining the good conditions in their service in the hospital.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.9 no.1
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• pp.98-104
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• 1998

A 12-year-old girl with a 6 year history of childhood-onset schizophrenia required 2 hospitalizations and long-term clozapine trial due to inadequate responses to combinations of typical neuroleptics and traditional treatments of schizophrenic disorder. On admission, she had continuous auditory and visual hallucinations, persecutory delusion, emotional instability, regression of behaviors including temper tantrums as well as specific developmental delays in learning, language, and motor coordination. The clozapine trial significantly reduced most of the positive symptoms, and facilitated in successful discharge from the hospital. During the 4 year clozapine treatment, no significant adverse reactions were noted, and she returned to a structured school setting with minimal degrees of schizophrenic symptoms. From this clinical experience, we suggest that clozapine might be safe and effective in treating treatment-refractory schizophrenic children.